PPT Neurological syndromes in systemic diseases and general medicine
Marta Lipowska

Encephalopathy
•Brain pathology of varies origins (metabolic, degenerative, genetic, posttraumatic, ischemic, hypoxic…)
•Generally it affects large parts of the brain instead of leading to identifiable focal changes
•Often developes when the reason is outside the brain

Encephalopathy
•Deprivation of oxygen (hypoxia, diffuse ischemia), substrate (hypoglycemia) or metabolic cofactor (thiamine deficiency)
•Diseases of organs other than the brain:
–Liver (hepatic coma)
–Kidney (uremia)
–Lung (CO2 narcosis)
–Pancreas
–Pituitary
–Thyroid (myxedema, thyrotoxicosis)
–Adrenal
•Exogenous poisons
•Abnormalities of ionic or acid-base environment
•Disordered temperature regulation (hypothermia, heat stroke)
•Primary neuronal or glial disorders

Ischemia Hypoxia Metabolic abnormalites
Brain dysfunction
headache,
disorder of consciousness:
(drowsiness®confusion®, stupor®coma)
convulsions,
involuntary movements (mioclonic)
focal neurological syndromes

Glasgow coma scale
Estimates the level of consciousness
Scores:
Eye opening response (1-4 points)
Verbal response (1-5 points)
Motor response (1-6 points)
3-8 points -coma

Seizures (convulsions) as a part of ecephalopathy
•Metabolic abnormalities: glucose¯, Na¯, Ca¯, Mg¯
•Toxic factors („irritating”)
•Drugs (neuroleptics)
•Acute brain disorders (stroke, inflammation, trauma)

a patient with pneumonia
(secondary CNS hypoxia)
confusion

A patient with encephalopathy-how to diagnose?
Blood and urine tests
Chest X-ray, other pulmonary investigations
Cardiological investigations etc.
Sometimes
Neurological examinations:
CT scan, MRI, CSF

•Brain MRI scan in metabolic encephalopathy (renal failure)?

•Brain MRI scan in metabolic encephalopathy (renal failure)?
•EEG reflects level of consciousness (changes in the EEG correlate with the severity of cerebral dysfunction)

EEG
•Alert
•drowsy
•lethargic
•stuporous
•comatose
•brain dead
•Normal EEG
•Different abnormalities
•Electrocerebral silence
Worse ence-phalo-pathy

Peripheral aquired neuropathies
•metabolic (diabetic, uremic)
•associated with dietary states
•Vasculitic (usually associated with systemic diseases)
•Acromegalic, hypo-hyperthyroid
•paraneoplastic
•paraproteinemic

Signs and symptoms of neuropathy
•Motor deficit-flaccid weakness
•Sensory deficit-pain, paresthesia, sensory loss, ataxia
•Autonomic involvement

Distribution of neuropathy
•Polyneuropathy-symetric, diffuse lesions of peripheral nerves, distal
•Mononeuropathy-disorder of a single nerve
•Mononeuropathy multiplex –focal involvement of two or more nerves

Cardiovascular disorders
•Brain ischemia during:
–Heart attack
–Arrhythmia
–Blood pressure ­or ¯
–Heart failure
•Ischemic stroke with embolic material form heart:
–Artrial fibrillation
–Aortic or valve mitral disease, specially with bacterial vegetation
–Bacterial miocarditis
•Hypertensive encephalopathy

Lung diseases
•Pneumonia
–hipoxia
–Risk of bacterial meningitis
•Lung cancer
–Paraneoplastic syndromes
–Metastases
–Pancoast`s syndrome –superior sulcus lungs tumors –brachial plexus damage
•Chronic pulmonary insufficiency
–Noctural headaches

Renal diseases-renal failure
•Uremic encephalopathy
•Uremic neuropathy
•Dialysis dysequilibrium syndrome (usually seen with rapid dialysis at the onset of dialysis program)
–Headache, nausea, muscle cramps, rarely convulsion, delirium
•Neurologic complication of renal transplantation
–CNS lymphoma, unusual infections

Hematologic diseases
•Leukemias
–Hemorrhage socondary to thrombocytopenia
–Infections secondary to low white blood cells counts
–Tumors of CNS
–Meningeal invasion (of any acute lymphoma)
–Complications of chemiotherapy

Hematologic diseases
•Polycythemia
–Large nad small vessel cerebral infaction (hyperviscosity)
–Hemorrhages socondary to thrombocytopenia
•Platelet disorders (primary or secondary)
–infarctions and hemorrhages
•Disorders of coagulation (deficiency of ATIII, factors involved in plasma coagulation)
–Ischemic stroke in young patiens

POEMS syndrome
•P-polyneuropathy
•O-organomegaly
•E-endocrynopathy
•M-monoclonal protein
•S-skin changes (angiomas, hiperpigmentation)
–In the course of solitary osteosclerotic plasmocytoma

Endocrine diseases
Thyroid –Hypothyroidism
•Changes in cognition and level of consciousness ®myxedema coma
•Entrapment neuropathy-carpal tunnel syndrome (attributed to the accumulation of acid mucopolysaccarides in the nerve and surranding tissues)

Endocrine diseases
Thyroid -hyperthyroidism
•Ophalmoplegia (Graves disease)
•Thyreotoxic myopathy –weakness, wasting of the muscles, myasthenic signs
•Thyreotoxic periodic paralysis
•Irritation, anxiety

Endocrine diseases
Hypoparathyroidism
•Symptoms of hypocalcemia
–tetany –muscle spasms, paresthesia
–Convulsions
–Irritation, insomia

Diabetes mellitus
Diabetic neuropathy-occurs in 80% of patients
•Distal symetric, predominantly sensory, with or without autonomic manifestations
(the main problem-pain)
•Mononeuropathies
–Liability to pressure palsies (CTS)
–Cranial nerve palsies

qother factors
Neurogenic diabetic foot-pathogenesis
qpain and temperature sensory loss –susceptibility to injures

qautonomic nerves involvement –dry skin, vascular autoregulation disturbences
Garland`s syndrome -femoral nerve damage (diabetic focal neuropathy)

Diabetes mellitus
•stroke risk factor
•hypo-, hyperglicemia:
–decrease of consciousness ®coma
–Convulsions
–Stroke-like episodes (focal syndromes)

Liver diseases
•Chronic liver disease, when portal hypertension induces an excessive portal collateral circulation ®cerebral intoxication
Encepalopathy, hepatic coma

Viral hepatitis type C-extrahepatic manifestations
•Cryoglobulinemia



•Peripheral neuropathies

Vitamin B12 (cyjanocobalamin) deficiency
•Megaloblastic anemia
•Classic clinical syndrome of subacute degeneration of spinal cord and polineuropathy

Neurologic paraneoplastic syndromes
•Results from indirect effect of primary malignancy on the nervous system (or other organs)
•Usually autoimmune cause:
The host makes antibodies against antigens in the neoplasm and that these antibodies cross-react with neural antigens
(anti-onconeural antibodies, anty –Hu)

Neurologic paraneoplastic syndromes
•Usually precede primary neoplasm occurence !!! (the most strong immunologic reaction at the beginning)
•Some patients may have easy controlled neoplasm but die from the neurologic disorder

Neurologic paraneoplastic syndromes
May affect any part of peripheral and central nervous system
•Encephalomyelitis
•Limbic encephalitis
•Cerebellar degeneration
•Opsoclonus
•Sensory neuropathy
•Myasthenic Lambert-Eaton syndrome
•Dermatomyositis and polymyositis

Treatment
•Treatment of underlying malignancy
•Immunomodulatory therapy

Collagen-vascular diseases
•Periarteritis nodosa
•systemic lupus erythematosus,
•rheumatoid arthritis,
•Wegener granulomatosis
•Churg-Strauss vasculitis
Vasculitis syndromes

•Vasculitis of central and peripheral vessels of nervous system
•Encephalopathy-multiple infarcts
•Vasculitic neuropathy

Alcohol abuse
•Acute alcohol intoxication –decrease of consciousness (euphoria/dysphoria, slurred speech and ataxic gait, stupor ® coma)
•Ethanol withdrawal syndromes (in dependency) :
tremor, hallucinosis, seizures®delirium tremens

Chronic alcohol abuse
•Wernicke-Korsakoff syndrome -Thiamine deficiency : dementia, amnesia, sometimes with confabulation
•Alcoholic cerebellar degeneration
•Alcoholic neuropathy, myopathy
•head trauma
•Complications of head trauma+withdrawal syndromes-„alcoholic” epilepsy

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